POS1573 LONG-TERM EFFICACY AND SAFETY OF CANAKINUMAB IN PATIENTS WITH TRAPS (TUMOR NECROSIS FACTOR RECEPTOR-ASSOCIATED PERIODIC SYNDROME) - INTERIM ANALYSIS OF THE RELIANCE REGISTRY
Authors: N. Blank, C. Schuetz, M. Hufnagel, G. Horneff, M. Borte, T. Kallinich, P. Oommen, A. Janda, J. Henes, J. Weber-Arden, J. B. Kuemmerle-Deschner
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Autoinflammatory periodic fever syndromes are characterized by severe systemic and organ inflammation as well as high burden for the patients and their families. Successful treatment was achieved in clinical trials and in real life with the interleukin-1β inhibitor canakinumab (CAN) leading to rapid remission of symptoms and normalization of laboratory parameters in most patients. <h3>Objectives</h3> The present study explores the long-term efficacy and safety of CAN under routine clinical practice conditions in pediatric (age ≥2 years) and adult patients with autoinflammatory periodic fever syndromes including TRAPS (tumor necrosis factor receptor-associated periodic syndrome). <h3>Methods</h3> RELIANCE is a prospective, non-interventional, multi-center, observational study based in Germany. Patients with clinically confirmed diagnoses of TRAPS who routinely receive CAN are enrolled to evaluate efficacy and safety of CAN under standard clinical practice conditions. Disease remission by physicians´ assessment, disease activity and fatigue by patients' assessment, days absent from school/work due to study indication, and inflammatory markers were assessed at baseline and at 6-monthly intervals. <h3>Results</h3> The interim analysis of TRAPS patients enrolled by December 2022 includes data from baseline (N=21), 18- (N=12) and 30-month visits (N=10). At baseline, median age was 15 years (3−43 years) and median duration of prior CAN treatment was 1 year (0−4 years). The proportion of female patients was 67%. At month 30, 3 patients received standard dose CAN, 4 patients greater than standard dose CAN and 2 patients lower than standard dose CAN. Preliminary results indicate stable remission by physicians´ assessment (in 62.5 % of patients), low disease activity by patient´s assessment (1.5 out of 10) and inflammatory markers within normal limits (<b>Table 1</b>). However, a fatigue score of 4 out of 10 might contribute to the impairment of social life reported by patients. One patient was affected by N=9 serious adverse events, none of which was classified as drug related. <h3>Conclusion</h3> The present interim data from TRAPS patients indicate that long-term CAN treatment is safe and effective. Patient reported fatigue and impairment of social life leave room for further improvement. Further RELIANCE interim and end-of-study data will be analyzed to assess efficacy and safety of long-term CAN treatment in patients with TRAPS. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> Norbert Blank Consultant of: Novartis, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Actelion, UCB, Boehringer-Ingelheim, Roche, Grant/research support from: Novartis, Sobi, Catharina Schuetz Grant/research support from: Novartis, Markus Hufnagel Grant/research support from: Novartis, Gerd Horneff Speakers bureau: AbbVie, Bayer, Chugai, Merck Sharp &amp; Dohme, Novartis, Pfizer, Roche, Grant/research support from: AbbVie, Chugai, Merck Sharp &amp; Dohme, Novartis, Pfizer, Roche, Michael Borte Grant/research support from: Pfizer, Shire, Tilmann Kallinich Speakers bureau: Roche, Prasad Oommen Grant/research support from: Novartis, Ales Janda: None declared, Jörg Henes Consultant of: Novartis, AbbVie, Sobi, Roche, Janssen, Boehringer-Ingelheim, Grant/research support from: Novartis, Roche, Julia Weber-Arden Employee of: Novartis, J. B. Kuemmerle-Deschner Consultant of: Novartis, AbbVie, Sobi, Grant/research support from: Novartis, AbbVie, Sobi.